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一名患有3q25.1-q25.32区域8.9 Mb新发间质性缺失的儿童出现发育迟缓及面部畸形:3q25缺失综合征的基因型-表型相关性

Developmental delay and facial dysmorphism in a child with an 8.9 Mb de novo interstitial deletion of 3q25.1-q25.32: Genotype-phenotype correlations of chromosome 3q25 deletion syndrome.

作者信息

Moortgat Stephanie, Verellen-Dumoulin Christine, Maystadt Isabelle, Parmentier Benoit, Grisart Bernard, Hennecker Jean-Luc, Destree Anne

机构信息

Centre de Génétique Humaine, Institut de Pathologie et de Génétique, Charleroi (Gosselies), Belgium.

出版信息

Eur J Med Genet. 2011 Mar-Apr;54(2):177-80. doi: 10.1016/j.ejmg.2010.11.011. Epub 2010 Dec 15.

Abstract

Interstitial deletions of the long arm of chromosome 3 are rare and detailed genotype-phenotype correlations are not well established. We report on the clinical, cytogenetic and molecular findings of a 5-year-old patient with a de novo interstitial deletion from 3q25.1 to 3q25.32. Clinical features include relative microcephaly, developmental delay and facial dysmorphism with a coarse face, ptosis, synophrys, epicanthic folds, broad nasal bridge, long philtrum, large mouth with full lips, dysplastic and low-set ears. Revealed by conventional banding techniques, the deleted region of 8.9 Mb was confirmed by fluorescent in situ hybridization (FISH) analyses and array comparative genomic hybridization (array-CGH). To our knowledge, this is the smallest interstitial deletion reported in the 3q25 region. The phenotype of our patient is compared with the 10 previously reported cases implicating the 3q25 region.

摘要

3号染色体长臂的间质性缺失较为罕见,详细的基因型-表型相关性尚未完全确立。我们报告了一名5岁患者的临床、细胞遗传学和分子学检查结果,该患者存在新发的3q25.1至3q25.32间质性缺失。临床特征包括相对小头畸形、发育迟缓以及面部畸形,表现为面容粗糙、上睑下垂、连眉、内眦赘皮、鼻梁宽、人中长、嘴巴大且嘴唇丰满、耳部发育异常及位置较低。通过传统显带技术发现的8.9 Mb缺失区域,经荧光原位杂交(FISH)分析和阵列比较基因组杂交(array-CGH)得以证实。据我们所知,这是3q25区域报道的最小间质性缺失。我们将该患者的表型与之前报道的涉及3q25区域的10例病例进行了比较。

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